The Experts below are selected from a list of 10713 Experts worldwide ranked by ideXlab platform
Michael Joseph Krowka - One of the best experts on this subject based on the ideXlab platform.
-
relationship between portopulmonary hypertension and splenectomy mayo clinic experience and review of published works
Hepatology Research, 2018Co-Authors: Justin M. Segraves, Michael D. Leise, Rodrigo Cartinceba, Michael Joseph KrowkaAbstract:AIM: Portopulmonary hypertension is a serious complication of portal hypertension that can lead to right heart failure and death. To our knowledge, an association between portopulmonary hypertension and prior splenectomy has not been described previously. The goals of this study were to describe the frequency of splenectomy in portopulmonary hypertension and compare selected parameters between portopulmonary hypertension subgroups. METHODS: This is a retrospective analysis of patients diagnosed with portopulmonary hypertension between 1 January 1988 and 30 June 2015 at Mayo Clinic (Rochester, MN, USA). We compared age, sex, right ventricle systolic pressure by echocardiography, and right heart catheterization measurements/calculations among subgroups of portopulmonary hypertension patients with splenectomy and/or Autoimmune Liver Disease (Autoimmune hepatitis/primary biliary cirrhosis/primary sclerosing cholangitis). RESULTS: The cohort consisted of 141 patients, of whom 8 (6%) had a history of splenectomy prior to the development of portopulmonary hypertension. Twenty-seven (19%) portopulmonary hypertension patients had Autoimmune Liver Disease, and 5 of 8 (62.5%) splenectomized portopulmonary hypertension patients had Autoimmune Liver Disease. No significant difference was noted in right heart catheterization measurements/calculations between splenectomized and non-splenectomized portopulmonary hypertension patients. Right ventricle systolic pressure by echocardiography was significantly higher in those splenectomized. CONCLUSIONS: Prior history of splenectomy in portopulmonary hypertension was 6% in this cohort. The combination of Autoimmune Liver Disease and splenectomy in portopulmonary hypertension was not uncommon. History of splenectomy in patients with portal hypertension and/or Autoimmune Liver Disease may have clinical implications.
-
Relationship between portopulmonary hypertension and splenectomy: Mayo Clinic experience and review of published works: Portopulmonary hypertension and splenectomy
Hepatology Research, 2017Co-Authors: Justin M. Segraves, Rodrigo Cartin-ceba, Michael D. Leise, Michael Joseph KrowkaAbstract:AIM: Portopulmonary hypertension is a serious complication of portal hypertension that can lead to right heart failure and death. To our knowledge, an association between portopulmonary hypertension and prior splenectomy has not been described previously. The goals of this study were to describe the frequency of splenectomy in portopulmonary hypertension and compare selected parameters between portopulmonary hypertension subgroups. METHODS: This is a retrospective analysis of patients diagnosed with portopulmonary hypertension between 1 January 1988 and 30 June 2015 at Mayo Clinic (Rochester, MN, USA). We compared age, sex, right ventricle systolic pressure by echocardiography, and right heart catheterization measurements/calculations among subgroups of portopulmonary hypertension patients with splenectomy and/or Autoimmune Liver Disease (Autoimmune hepatitis/primary biliary cirrhosis/primary sclerosing cholangitis). RESULTS: The cohort consisted of 141 patients, of whom 8 (6%) had a history of splenectomy prior to the development of portopulmonary hypertension. Twenty-seven (19%) portopulmonary hypertension patients had Autoimmune Liver Disease, and 5 of 8 (62.5%) splenectomized portopulmonary hypertension patients had Autoimmune Liver Disease. No significant difference was noted in right heart catheterization measurements/calculations between splenectomized and non-splenectomized portopulmonary hypertension patients. Right ventricle systolic pressure by echocardiography was significantly higher in those splenectomized. CONCLUSIONS: Prior history of splenectomy in portopulmonary hypertension was 6% in this cohort. The combination of Autoimmune Liver Disease and splenectomy in portopulmonary hypertension was not uncommon. History of splenectomy in patients with portal hypertension and/or Autoimmune Liver Disease may have clinical implications.
-
the relationship between portopulmonary hypertension and splenectomy mayo clinic experience and literature review
Hepatology Research, 2017Co-Authors: Justin M. Segraves, Michael D. Leise, Rodrigo Cartinceba, Michael Joseph KrowkaAbstract:Background & Aims Portopulmonary hypertension is a serious complication of portal hypertension that can lead to right heart failure and death. To our knowledge, an association between portopulmonary hypertension and prior splenectomy has not been described previously. The goals of this study were to describe the frequency of splenectomy in portopulmonary hypertension and compare selected parameters between portopulmonary hypertension subgroups. Methods Retrospective analysis of patients diagnosed with portopulmonary hypertension between 01/01/1988 – 06/30/2015 at Mayo Clinic, Rochester, MN. Compared age, sex, right ventricle systolic pressure by echocardiography, and right heart catheterization measurements/calculations among subgroups of portopulmonary hypertension patients with splenectomy and/or Autoimmune Liver Disease (Autoimmune hepatitis/primary biliary cirrhosis/primary sclerosing cholangitis). Results Cohort of 141 patients. 8 (6%) with history of splenectomy prior to the development of portopulmonary hypertension. 27 (19%) portopulmonary hypertension patients had Autoimmune Liver Disease; 5/8 (62.5%) of splenectomized portopulmonary hypertension patients had Autoimmune Liver Disease. No significant difference was noted in right heart catheterization measurements/calculations between splenectomized and non splenectomized portopulmonary hypertension patients. Right ventricle systolic pressure by echocardiography was significantly higher in those splenectomized. Conclusions Prior history of splenectomy in portopulmonary hypertension was 6% in this cohort. The combination of Autoimmune Liver Disease and splenectomy in portopulmonary hypertension was not uncommon. The history of splenectomy in patients with portal hypertension and/or Autoimmune Liver may have clinical implications.
Giorgina Mielivergani - One of the best experts on this subject based on the ideXlab platform.
-
seamless management of juvenile Autoimmune Liver Disease long term medical and social outcome
The Journal of Pediatrics, 2020Co-Authors: Angelo Di Giorgio, Nedim Hadzic, Anil Dhawan, Maesha Deheragoda, Michael A Heneghan, D Vergani, Giorgina Mielivergani, Marianne SamynAbstract:Objectives To report baseline features and long-term medical/social outcomes of juvenile Autoimmune Liver Disease, including Autoimmune hepatitis (AIH) and Autoimmune sclerosing cholangitis (ASC), managed in a single tertiary center. Study design Retrospective study of children diagnosed in 2000-2004 with AIH/ASC followed up to date. Patients with abnormal cholangiogram were classified as ASC. Presentation and outcome features were compared. Results Eighty-three children were included (42 female, median age 12.1 years [8.5-14.1 years], AIH = 54, ASC = 29). Most (65%) had antinuclear and/or anti-smooth muscle autoantibodies; 6% presented with acute Liver failure; 29% had histologic evidence of cirrhosis. The 1999 and simplified International Autoimmune Hepatitis Group criteria failed to diagnose up to 26% of patients with AIH and 48% with ASC, and the proposed the European Society for Pediatric Gastroenterology, Hepatology and Nutrition criteria were accurate. Response to treatment was excellent with 95% achieving normal transaminase levels. During follow-up, 31% had at least 1 relapse episode; 3 patients with AIH developed cholangiopathy and 5 patients with ASC developed progressive bile duct injury. At last follow-up (median of 14.5 years, 10.4-16.8), 99% were alive, 11 underwent transplantation and 1 is listed for transplant. Five-, 10-, and 15-year transplant-free survival rates were 95%, 88%, and 83%; patients with ASC and those relapsing being more likely to require transplant. Social outcome was excellent with 93% in employment/education. Conclusions Seamless management of juvenile Autoimmune Liver Disease leads to excellent clinical and social outcomes. Despite good response to immunosuppressive treatment, patients with ASC have a worse prognosis than those with AIH. Diagnostic models developed for adults are unsatisfactory to correctly diagnose juvenile Autoimmune Liver Disease.
-
ptu 010 role for gpr15 rather than beta 7 integrins in the pathogenesis of Autoimmune Liver Disease
Gut, 2019Co-Authors: Jonathon Graham, D Vergani, Giorgina Mielivergani, Rodrigo Liberal, Suj Mukherjee, Muhammed Yuksel, Bu HayeeAbstract:Introduction G protein-coupled receptor 15 (GPR15) is a chemoattractant receptor that directs homing of lymphocytes to the colon. Furthermore, it has been shown to be a mediator of effector T cell homing during intestinal inflammation. Evidence exists showing infiltration of gut derived α4β7+ and CCR9+ T cells in the hepatic infiltrate of patients with Autoimmune Liver Disease (AILD), in particular primary sclerosing cholangitis (PSC), with expression of their complementary ligands also being identified on the hepatic endothelium. However, the role of GPR15 in hepatic gut T cell homing remains to be defined. Methods Explanted Liver tissue was collected from patients undergoing orthotopic Liver transplantation for chronic Liver Disease (Alcoholic Liver Disease [ALD] n=3, Non-alcoholic fatty Liver Disease [NAFLD] n=3, PSC n=4) with healthy control tissue sourced from patients undergoing hepatocellular carcinoma (HCC) resection (n=4). Liver infiltrating lymphocytes (LIL) were isolated using a mechanical homogenisation and centrifugation/filtration technique. Expression of gut homing markers on T cells was quantified using flow cytometry. Results Expression of GPR15 was significantly increased on CD4+ effector T cells (CD4+ CD127+ CD45RO+) in NAFLD, ALD and PSC patients compared to healthy controls (9.7 ± 0.4, 12.7 ± 0.9 and 20.1 ± 1.8 vs 4.1 ± 1.8, P Conclusions For some time, evidence pointed towards β7 integrins as the drivers of the hepatic inflammation seen in patients with AILD and associated inflammatory bowel Disease as a result of aberrant effector T cells homing from the gut to the Liver. These data reveal a somewhat redundant role for β7 integrins, which could be regarded as a non-Disease specific feature of advanced Disease, and highlight GPR15 as the primary mediator of effector gut T cell homing.
-
the role of invariant nkt in Autoimmune Liver Disease can vitamin d act as an immunomodulator
Canadian Journal of Gastroenterology & Hepatology, 2018Co-Authors: Marco Lenzi, Daniel S. Smyk, Dimitrios P. Bogdanos, D Vergani, Giorgina Mielivergani, Athanasios MavropoulosAbstract:Natural killer T (NKT) cells are a distinct lineage of T cells which express both the T cell receptor (TCR) and natural killer (NK) cell markers. Invariant NKT (iNKT) cells bear an invariant TCR and recognize a small variety of glycolipid antigens presented by CD1d (nonclassical MHC-I). CD1d-restricted iNKT cells are regulators of immune responses and produce cytokines that may be proinflammatory (such as interferon-gamma (IFN-γ)) or anti-inflammatory (such as IL-4). iNKT cells also appear to play a role in B cell regulation and antibody production. Alpha-galactosylceramide (α-GalCer), a derivative of the marine sponge, is a potent stimulator of iNKT cells and has been proposed as a therapeutic iNKT cell activator. Invariant NKT cells have been implicated in the development and perpetuation of several Autoimmune Diseases such as multiple sclerosis and systemic lupus erythematosus (SLE). Animal models of SLE have shown abnormalities in iNKT cells numbers and function, and an inverse correlation between the frequency of NKT cells and IgG levels has also been observed. The role of iNKT cells in Autoimmune Liver Disease (AiLD) has not been extensively studied. This review discusses the current data with regard to iNKT cells function in AiLD, in addition to providing an overview of iNKT cells function in other Autoimmune conditions and animal models. We also discuss data regarding the immunomodulatory effects of vitamin D on iNKT cells, which may serve as a potential therapeutic target, given that deficiencies in vitamin D have been reported in various Autoimmune disorders.
-
real world management of juvenile Autoimmune Liver Disease
United European gastroenterology journal, 2018Co-Authors: Y S De Boer, D Vergani, Giorgina Mielivergani, Rodrigo LiberalAbstract:Background and aims: Juvenile Autoimmune Liver Disease (JAILD) includes paediatric forms of Autoimmune hepatitis (AIH) and Autoimmune sclerosing cholangitis (ASC). Since evidence is scarce, there are currently no evidence-based management guidelines for juvenile AIH. This survey was carried out amongst the paediatric members of the International AIH Group (IAIHG) to describe their practices in the management of JAILD. Methods: An online survey questionnaire was distributed to members of the IAIHG with active practice (https://www.surveymonkey.de/r/Juvenile_AILD). The questionnaire consisted of four clinical scenarios on different presentations of AIH. Results: Fifty-eight surveys were sent to the IAIHG members, out of which 43 (74%, 22 countries, four continents) were returned. None reported budesonide as a first-line induction agent for the acute presentation of AIH. Sixteen (37%) routinely perform Liver biopsy at three years of biochemical remission. Thirty-five respondents (81%) perform magnetic resonance cholangiography (MRC) at presentation. Ciclosporin is the most widely used second-line agent (number of patients treated = ∼360, 21 centres). Mycophenolate mofetil (n = ∼225, 31 centres), tacrolimus (n = ∼130, 21 centres) and sirolimus (n = ∼5, 3 centres) are less often reported. Rescue therapy with infliximab and rituximab has been tried in eight centres (n = ∼19) and nine centres (n = ∼16), respectively. Conclusions: Prednisolone remains the preferred first-line induction agent in JAILD. MRC at presentation is performed by the large majority of participants. Participants reported a wide variation in performing Liver biopsy for therapy evaluation during follow-up. Within the paediatric members of the IAIHG there is considerable experience with second-line therapeutic agents.
-
anti tnfα treatment in children and adolescents with combined inflammatory bowel Disease and Autoimmune Liver Disease
Journal of Pediatric Gastroenterology and Nutrition, 2018Co-Authors: Natalia Nedelkopoulou, D Vergani, Babu Vadamalayan, Giorgina MieliverganiAbstract:ABSTRACTObjectives:Inflammatory bowel Disease (IBD) and Autoimmune Liver Disease (AILD) are closely associated, the former often dictating progression of the latter. Antibodies to tumor necrosis factor alpha (anti-TNFα) are effective in the management of IBD, but may cause Liver injury.Methods:Retro
Justin M. Segraves - One of the best experts on this subject based on the ideXlab platform.
-
relationship between portopulmonary hypertension and splenectomy mayo clinic experience and review of published works
Hepatology Research, 2018Co-Authors: Justin M. Segraves, Michael D. Leise, Rodrigo Cartinceba, Michael Joseph KrowkaAbstract:AIM: Portopulmonary hypertension is a serious complication of portal hypertension that can lead to right heart failure and death. To our knowledge, an association between portopulmonary hypertension and prior splenectomy has not been described previously. The goals of this study were to describe the frequency of splenectomy in portopulmonary hypertension and compare selected parameters between portopulmonary hypertension subgroups. METHODS: This is a retrospective analysis of patients diagnosed with portopulmonary hypertension between 1 January 1988 and 30 June 2015 at Mayo Clinic (Rochester, MN, USA). We compared age, sex, right ventricle systolic pressure by echocardiography, and right heart catheterization measurements/calculations among subgroups of portopulmonary hypertension patients with splenectomy and/or Autoimmune Liver Disease (Autoimmune hepatitis/primary biliary cirrhosis/primary sclerosing cholangitis). RESULTS: The cohort consisted of 141 patients, of whom 8 (6%) had a history of splenectomy prior to the development of portopulmonary hypertension. Twenty-seven (19%) portopulmonary hypertension patients had Autoimmune Liver Disease, and 5 of 8 (62.5%) splenectomized portopulmonary hypertension patients had Autoimmune Liver Disease. No significant difference was noted in right heart catheterization measurements/calculations between splenectomized and non-splenectomized portopulmonary hypertension patients. Right ventricle systolic pressure by echocardiography was significantly higher in those splenectomized. CONCLUSIONS: Prior history of splenectomy in portopulmonary hypertension was 6% in this cohort. The combination of Autoimmune Liver Disease and splenectomy in portopulmonary hypertension was not uncommon. History of splenectomy in patients with portal hypertension and/or Autoimmune Liver Disease may have clinical implications.
-
Relationship between portopulmonary hypertension and splenectomy: Mayo Clinic experience and review of published works: Portopulmonary hypertension and splenectomy
Hepatology Research, 2017Co-Authors: Justin M. Segraves, Rodrigo Cartin-ceba, Michael D. Leise, Michael Joseph KrowkaAbstract:AIM: Portopulmonary hypertension is a serious complication of portal hypertension that can lead to right heart failure and death. To our knowledge, an association between portopulmonary hypertension and prior splenectomy has not been described previously. The goals of this study were to describe the frequency of splenectomy in portopulmonary hypertension and compare selected parameters between portopulmonary hypertension subgroups. METHODS: This is a retrospective analysis of patients diagnosed with portopulmonary hypertension between 1 January 1988 and 30 June 2015 at Mayo Clinic (Rochester, MN, USA). We compared age, sex, right ventricle systolic pressure by echocardiography, and right heart catheterization measurements/calculations among subgroups of portopulmonary hypertension patients with splenectomy and/or Autoimmune Liver Disease (Autoimmune hepatitis/primary biliary cirrhosis/primary sclerosing cholangitis). RESULTS: The cohort consisted of 141 patients, of whom 8 (6%) had a history of splenectomy prior to the development of portopulmonary hypertension. Twenty-seven (19%) portopulmonary hypertension patients had Autoimmune Liver Disease, and 5 of 8 (62.5%) splenectomized portopulmonary hypertension patients had Autoimmune Liver Disease. No significant difference was noted in right heart catheterization measurements/calculations between splenectomized and non-splenectomized portopulmonary hypertension patients. Right ventricle systolic pressure by echocardiography was significantly higher in those splenectomized. CONCLUSIONS: Prior history of splenectomy in portopulmonary hypertension was 6% in this cohort. The combination of Autoimmune Liver Disease and splenectomy in portopulmonary hypertension was not uncommon. History of splenectomy in patients with portal hypertension and/or Autoimmune Liver Disease may have clinical implications.
-
the relationship between portopulmonary hypertension and splenectomy mayo clinic experience and literature review
Hepatology Research, 2017Co-Authors: Justin M. Segraves, Michael D. Leise, Rodrigo Cartinceba, Michael Joseph KrowkaAbstract:Background & Aims Portopulmonary hypertension is a serious complication of portal hypertension that can lead to right heart failure and death. To our knowledge, an association between portopulmonary hypertension and prior splenectomy has not been described previously. The goals of this study were to describe the frequency of splenectomy in portopulmonary hypertension and compare selected parameters between portopulmonary hypertension subgroups. Methods Retrospective analysis of patients diagnosed with portopulmonary hypertension between 01/01/1988 – 06/30/2015 at Mayo Clinic, Rochester, MN. Compared age, sex, right ventricle systolic pressure by echocardiography, and right heart catheterization measurements/calculations among subgroups of portopulmonary hypertension patients with splenectomy and/or Autoimmune Liver Disease (Autoimmune hepatitis/primary biliary cirrhosis/primary sclerosing cholangitis). Results Cohort of 141 patients. 8 (6%) with history of splenectomy prior to the development of portopulmonary hypertension. 27 (19%) portopulmonary hypertension patients had Autoimmune Liver Disease; 5/8 (62.5%) of splenectomized portopulmonary hypertension patients had Autoimmune Liver Disease. No significant difference was noted in right heart catheterization measurements/calculations between splenectomized and non splenectomized portopulmonary hypertension patients. Right ventricle systolic pressure by echocardiography was significantly higher in those splenectomized. Conclusions Prior history of splenectomy in portopulmonary hypertension was 6% in this cohort. The combination of Autoimmune Liver Disease and splenectomy in portopulmonary hypertension was not uncommon. The history of splenectomy in patients with portal hypertension and/or Autoimmune Liver may have clinical implications.
D Vergani - One of the best experts on this subject based on the ideXlab platform.
-
seamless management of juvenile Autoimmune Liver Disease long term medical and social outcome
The Journal of Pediatrics, 2020Co-Authors: Angelo Di Giorgio, Nedim Hadzic, Anil Dhawan, Maesha Deheragoda, Michael A Heneghan, D Vergani, Giorgina Mielivergani, Marianne SamynAbstract:Objectives To report baseline features and long-term medical/social outcomes of juvenile Autoimmune Liver Disease, including Autoimmune hepatitis (AIH) and Autoimmune sclerosing cholangitis (ASC), managed in a single tertiary center. Study design Retrospective study of children diagnosed in 2000-2004 with AIH/ASC followed up to date. Patients with abnormal cholangiogram were classified as ASC. Presentation and outcome features were compared. Results Eighty-three children were included (42 female, median age 12.1 years [8.5-14.1 years], AIH = 54, ASC = 29). Most (65%) had antinuclear and/or anti-smooth muscle autoantibodies; 6% presented with acute Liver failure; 29% had histologic evidence of cirrhosis. The 1999 and simplified International Autoimmune Hepatitis Group criteria failed to diagnose up to 26% of patients with AIH and 48% with ASC, and the proposed the European Society for Pediatric Gastroenterology, Hepatology and Nutrition criteria were accurate. Response to treatment was excellent with 95% achieving normal transaminase levels. During follow-up, 31% had at least 1 relapse episode; 3 patients with AIH developed cholangiopathy and 5 patients with ASC developed progressive bile duct injury. At last follow-up (median of 14.5 years, 10.4-16.8), 99% were alive, 11 underwent transplantation and 1 is listed for transplant. Five-, 10-, and 15-year transplant-free survival rates were 95%, 88%, and 83%; patients with ASC and those relapsing being more likely to require transplant. Social outcome was excellent with 93% in employment/education. Conclusions Seamless management of juvenile Autoimmune Liver Disease leads to excellent clinical and social outcomes. Despite good response to immunosuppressive treatment, patients with ASC have a worse prognosis than those with AIH. Diagnostic models developed for adults are unsatisfactory to correctly diagnose juvenile Autoimmune Liver Disease.
-
ptu 010 role for gpr15 rather than beta 7 integrins in the pathogenesis of Autoimmune Liver Disease
Gut, 2019Co-Authors: Jonathon Graham, D Vergani, Giorgina Mielivergani, Rodrigo Liberal, Suj Mukherjee, Muhammed Yuksel, Bu HayeeAbstract:Introduction G protein-coupled receptor 15 (GPR15) is a chemoattractant receptor that directs homing of lymphocytes to the colon. Furthermore, it has been shown to be a mediator of effector T cell homing during intestinal inflammation. Evidence exists showing infiltration of gut derived α4β7+ and CCR9+ T cells in the hepatic infiltrate of patients with Autoimmune Liver Disease (AILD), in particular primary sclerosing cholangitis (PSC), with expression of their complementary ligands also being identified on the hepatic endothelium. However, the role of GPR15 in hepatic gut T cell homing remains to be defined. Methods Explanted Liver tissue was collected from patients undergoing orthotopic Liver transplantation for chronic Liver Disease (Alcoholic Liver Disease [ALD] n=3, Non-alcoholic fatty Liver Disease [NAFLD] n=3, PSC n=4) with healthy control tissue sourced from patients undergoing hepatocellular carcinoma (HCC) resection (n=4). Liver infiltrating lymphocytes (LIL) were isolated using a mechanical homogenisation and centrifugation/filtration technique. Expression of gut homing markers on T cells was quantified using flow cytometry. Results Expression of GPR15 was significantly increased on CD4+ effector T cells (CD4+ CD127+ CD45RO+) in NAFLD, ALD and PSC patients compared to healthy controls (9.7 ± 0.4, 12.7 ± 0.9 and 20.1 ± 1.8 vs 4.1 ± 1.8, P Conclusions For some time, evidence pointed towards β7 integrins as the drivers of the hepatic inflammation seen in patients with AILD and associated inflammatory bowel Disease as a result of aberrant effector T cells homing from the gut to the Liver. These data reveal a somewhat redundant role for β7 integrins, which could be regarded as a non-Disease specific feature of advanced Disease, and highlight GPR15 as the primary mediator of effector gut T cell homing.
-
the role of invariant nkt in Autoimmune Liver Disease can vitamin d act as an immunomodulator
Canadian Journal of Gastroenterology & Hepatology, 2018Co-Authors: Marco Lenzi, Daniel S. Smyk, Dimitrios P. Bogdanos, D Vergani, Giorgina Mielivergani, Athanasios MavropoulosAbstract:Natural killer T (NKT) cells are a distinct lineage of T cells which express both the T cell receptor (TCR) and natural killer (NK) cell markers. Invariant NKT (iNKT) cells bear an invariant TCR and recognize a small variety of glycolipid antigens presented by CD1d (nonclassical MHC-I). CD1d-restricted iNKT cells are regulators of immune responses and produce cytokines that may be proinflammatory (such as interferon-gamma (IFN-γ)) or anti-inflammatory (such as IL-4). iNKT cells also appear to play a role in B cell regulation and antibody production. Alpha-galactosylceramide (α-GalCer), a derivative of the marine sponge, is a potent stimulator of iNKT cells and has been proposed as a therapeutic iNKT cell activator. Invariant NKT cells have been implicated in the development and perpetuation of several Autoimmune Diseases such as multiple sclerosis and systemic lupus erythematosus (SLE). Animal models of SLE have shown abnormalities in iNKT cells numbers and function, and an inverse correlation between the frequency of NKT cells and IgG levels has also been observed. The role of iNKT cells in Autoimmune Liver Disease (AiLD) has not been extensively studied. This review discusses the current data with regard to iNKT cells function in AiLD, in addition to providing an overview of iNKT cells function in other Autoimmune conditions and animal models. We also discuss data regarding the immunomodulatory effects of vitamin D on iNKT cells, which may serve as a potential therapeutic target, given that deficiencies in vitamin D have been reported in various Autoimmune disorders.
-
real world management of juvenile Autoimmune Liver Disease
United European gastroenterology journal, 2018Co-Authors: Y S De Boer, D Vergani, Giorgina Mielivergani, Rodrigo LiberalAbstract:Background and aims: Juvenile Autoimmune Liver Disease (JAILD) includes paediatric forms of Autoimmune hepatitis (AIH) and Autoimmune sclerosing cholangitis (ASC). Since evidence is scarce, there are currently no evidence-based management guidelines for juvenile AIH. This survey was carried out amongst the paediatric members of the International AIH Group (IAIHG) to describe their practices in the management of JAILD. Methods: An online survey questionnaire was distributed to members of the IAIHG with active practice (https://www.surveymonkey.de/r/Juvenile_AILD). The questionnaire consisted of four clinical scenarios on different presentations of AIH. Results: Fifty-eight surveys were sent to the IAIHG members, out of which 43 (74%, 22 countries, four continents) were returned. None reported budesonide as a first-line induction agent for the acute presentation of AIH. Sixteen (37%) routinely perform Liver biopsy at three years of biochemical remission. Thirty-five respondents (81%) perform magnetic resonance cholangiography (MRC) at presentation. Ciclosporin is the most widely used second-line agent (number of patients treated = ∼360, 21 centres). Mycophenolate mofetil (n = ∼225, 31 centres), tacrolimus (n = ∼130, 21 centres) and sirolimus (n = ∼5, 3 centres) are less often reported. Rescue therapy with infliximab and rituximab has been tried in eight centres (n = ∼19) and nine centres (n = ∼16), respectively. Conclusions: Prednisolone remains the preferred first-line induction agent in JAILD. MRC at presentation is performed by the large majority of participants. Participants reported a wide variation in performing Liver biopsy for therapy evaluation during follow-up. Within the paediatric members of the IAIHG there is considerable experience with second-line therapeutic agents.
-
anti tnfα treatment in children and adolescents with combined inflammatory bowel Disease and Autoimmune Liver Disease
Journal of Pediatric Gastroenterology and Nutrition, 2018Co-Authors: Natalia Nedelkopoulou, D Vergani, Babu Vadamalayan, Giorgina MieliverganiAbstract:ABSTRACTObjectives:Inflammatory bowel Disease (IBD) and Autoimmune Liver Disease (AILD) are closely associated, the former often dictating progression of the latter. Antibodies to tumor necrosis factor alpha (anti-TNFα) are effective in the management of IBD, but may cause Liver injury.Methods:Retro
Michael D. Leise - One of the best experts on this subject based on the ideXlab platform.
-
relationship between portopulmonary hypertension and splenectomy mayo clinic experience and review of published works
Hepatology Research, 2018Co-Authors: Justin M. Segraves, Michael D. Leise, Rodrigo Cartinceba, Michael Joseph KrowkaAbstract:AIM: Portopulmonary hypertension is a serious complication of portal hypertension that can lead to right heart failure and death. To our knowledge, an association between portopulmonary hypertension and prior splenectomy has not been described previously. The goals of this study were to describe the frequency of splenectomy in portopulmonary hypertension and compare selected parameters between portopulmonary hypertension subgroups. METHODS: This is a retrospective analysis of patients diagnosed with portopulmonary hypertension between 1 January 1988 and 30 June 2015 at Mayo Clinic (Rochester, MN, USA). We compared age, sex, right ventricle systolic pressure by echocardiography, and right heart catheterization measurements/calculations among subgroups of portopulmonary hypertension patients with splenectomy and/or Autoimmune Liver Disease (Autoimmune hepatitis/primary biliary cirrhosis/primary sclerosing cholangitis). RESULTS: The cohort consisted of 141 patients, of whom 8 (6%) had a history of splenectomy prior to the development of portopulmonary hypertension. Twenty-seven (19%) portopulmonary hypertension patients had Autoimmune Liver Disease, and 5 of 8 (62.5%) splenectomized portopulmonary hypertension patients had Autoimmune Liver Disease. No significant difference was noted in right heart catheterization measurements/calculations between splenectomized and non-splenectomized portopulmonary hypertension patients. Right ventricle systolic pressure by echocardiography was significantly higher in those splenectomized. CONCLUSIONS: Prior history of splenectomy in portopulmonary hypertension was 6% in this cohort. The combination of Autoimmune Liver Disease and splenectomy in portopulmonary hypertension was not uncommon. History of splenectomy in patients with portal hypertension and/or Autoimmune Liver Disease may have clinical implications.
-
Relationship between portopulmonary hypertension and splenectomy: Mayo Clinic experience and review of published works: Portopulmonary hypertension and splenectomy
Hepatology Research, 2017Co-Authors: Justin M. Segraves, Rodrigo Cartin-ceba, Michael D. Leise, Michael Joseph KrowkaAbstract:AIM: Portopulmonary hypertension is a serious complication of portal hypertension that can lead to right heart failure and death. To our knowledge, an association between portopulmonary hypertension and prior splenectomy has not been described previously. The goals of this study were to describe the frequency of splenectomy in portopulmonary hypertension and compare selected parameters between portopulmonary hypertension subgroups. METHODS: This is a retrospective analysis of patients diagnosed with portopulmonary hypertension between 1 January 1988 and 30 June 2015 at Mayo Clinic (Rochester, MN, USA). We compared age, sex, right ventricle systolic pressure by echocardiography, and right heart catheterization measurements/calculations among subgroups of portopulmonary hypertension patients with splenectomy and/or Autoimmune Liver Disease (Autoimmune hepatitis/primary biliary cirrhosis/primary sclerosing cholangitis). RESULTS: The cohort consisted of 141 patients, of whom 8 (6%) had a history of splenectomy prior to the development of portopulmonary hypertension. Twenty-seven (19%) portopulmonary hypertension patients had Autoimmune Liver Disease, and 5 of 8 (62.5%) splenectomized portopulmonary hypertension patients had Autoimmune Liver Disease. No significant difference was noted in right heart catheterization measurements/calculations between splenectomized and non-splenectomized portopulmonary hypertension patients. Right ventricle systolic pressure by echocardiography was significantly higher in those splenectomized. CONCLUSIONS: Prior history of splenectomy in portopulmonary hypertension was 6% in this cohort. The combination of Autoimmune Liver Disease and splenectomy in portopulmonary hypertension was not uncommon. History of splenectomy in patients with portal hypertension and/or Autoimmune Liver Disease may have clinical implications.
-
the relationship between portopulmonary hypertension and splenectomy mayo clinic experience and literature review
Hepatology Research, 2017Co-Authors: Justin M. Segraves, Michael D. Leise, Rodrigo Cartinceba, Michael Joseph KrowkaAbstract:Background & Aims Portopulmonary hypertension is a serious complication of portal hypertension that can lead to right heart failure and death. To our knowledge, an association between portopulmonary hypertension and prior splenectomy has not been described previously. The goals of this study were to describe the frequency of splenectomy in portopulmonary hypertension and compare selected parameters between portopulmonary hypertension subgroups. Methods Retrospective analysis of patients diagnosed with portopulmonary hypertension between 01/01/1988 – 06/30/2015 at Mayo Clinic, Rochester, MN. Compared age, sex, right ventricle systolic pressure by echocardiography, and right heart catheterization measurements/calculations among subgroups of portopulmonary hypertension patients with splenectomy and/or Autoimmune Liver Disease (Autoimmune hepatitis/primary biliary cirrhosis/primary sclerosing cholangitis). Results Cohort of 141 patients. 8 (6%) with history of splenectomy prior to the development of portopulmonary hypertension. 27 (19%) portopulmonary hypertension patients had Autoimmune Liver Disease; 5/8 (62.5%) of splenectomized portopulmonary hypertension patients had Autoimmune Liver Disease. No significant difference was noted in right heart catheterization measurements/calculations between splenectomized and non splenectomized portopulmonary hypertension patients. Right ventricle systolic pressure by echocardiography was significantly higher in those splenectomized. Conclusions Prior history of splenectomy in portopulmonary hypertension was 6% in this cohort. The combination of Autoimmune Liver Disease and splenectomy in portopulmonary hypertension was not uncommon. The history of splenectomy in patients with portal hypertension and/or Autoimmune Liver may have clinical implications.
